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ScienceMED, vol. 4, 1.2013, 9-15, Bologna (Italy)
Chronic Cerebrospinal Venous Insufficiency
(CCSVI) IN Meniere Disease. Case or Cause?
Alpini D.C.(1), Bavera P.M.(2), Hahn A.(3), Mattei V.(1)
1) Sc. Institute S. Maria Nascente,“Don Carlo Gnocchi” Foundation, via Capecelatro 66, 20148 Milan, Italy
2) Medick-Up Angiology Center via Monte Rosa 13 tel. +39024985581, Milan, Italy
3) ENT dept. III Medical Faculty, Prague (Czech Republik)
Abstract
CCSVI is the acronym for Chronic Cerebrospinal Venous Insufficiency, initially
described by P.Zamboni, as being strongly associated with multiple sclerosis (MS).
It is a syndrome characterized by stenosis of the internal jugular veins (IJVs) and/or
azygous vein (AZ) with opening of collaterals and insufficient drainage.
Bavera PM carried out 823 Duplex exams on a control group of 60 patients without
MS. As expected CCSVI was found only in few subjects of the control group, three, two
females and one male, but all affected with Sudden Sensorineural Hearing Loss (SSHL).
Successively, we reported a case of bilateral SSHL with vertigo, showing evidence
of the CCSVI pattern at Duplex examination (not associated with MS). To the best
of the authors’ knowledge, this kind of association has never been reported.
We studied 52 patients affected with cochleo-vestibular disturbances subdivided
into two groups of out-patients:Definite unilateral Meniere (Men): 12 subjects (8
males and 4.females, mean age 41,6.yy) according to international AOO-HNS 1995
diagnostic criteria - No-Meniere (No-Men): 14 subjects (6.males and 8 females, mean
age 44,7.yy) affected with unilateral cocleo-vestibular impairment
A third group of subjects have been considered, as a “normal” group, 13 patients
(8 females and 5 males, mean age 45,5 yy) affected with Benign Paroxismal Positioning Vertigo (BPPV) with cochlear involvement
Asymmetrical artherious flow in VA or CA was revealed in 2 Men 9 no-Men and
1 BPPV, respectively 12,5 - 60,7 - and 8,6 %. Differences between Men and NoMen and between each of this group with respect to BPPV were highly significant
(p<0.001). Asymmetrical venous flow in IJV or VV was detected in 9 patients in
MEN group and in 4 in no-MEN and 2 BPPV, respectively 79 - 28,5 and 13 %.
Differences between Men and No-Men and between each of this group with respect
to BBV were highly significant (p<0.001)
Key words: CCSVI, Meniere Disease, Vertigo , Hearing loss
©2013 by MEDIMOND
Q419C0003
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Alpini D.C., Bavera P.M., Hahn A., Mattei V.
Introduction
As a matter of fact the venous outflow of the neck has aroused interest, and importance, mostly in these last three years after the so-called Zamboni method aimed
to detect and study “ Chronic Cerebrospinal Venous Insufficiency” (CCSVI) [1]. This
type of exam and problem, based on detection of abnormal venous outflow from the
head and neck, is principally linked with multiple sclerosis disease. It is characterised
by multiple stenoses of the extracranial venous draining pathways, i.e. the internal
jugular veins and the Azygous veins, which lead to collateral formation, alteration of
the blood-brain barrier. It could be diagnosed by means of MRI digital Venography
and, especially, Duplex examination of cerebral and cervical vein system. [2,3]
Abnormalities of cerebro-spinal venous flow have been described also in no-MS
patients. For example Koerte et al. [4] performed a 2D time-of-flight MR-venography
of the upper neck region to visualize the venous vasculature in patients with migraine.
They showed a higher prevalence of dense secondary extracranial venous networks
and a significantly larger percentage of venous outflow through secondary channels,
through epidural, vertebral, and deep cervical veins.
Bavera PM [5] carried out 823 Duplex exams on a control group of 60 patients
without MS. As expected CCSVI was found only in few subjects of the control
group, three, two females and one male, but all affected with Sudden Sensorineural
Hearing Loss (SSHL).
Successively, we reported [6] a case of bilateral SSHL with vertigo, showing
evidence of the CCSVI pattern at Duplex examination (not associated with MS). On
the basis of these experiences we decide to investigate Cerebrospinal Venous system
in Otoneurological patients even if not associated with Multiple Sclerosis.
In order to clarify the role of venous drainage in Meniere Disease we compared
cervico-cephalic emodynamic (arterial and venous flows) in three groups of patients
affected with vertigo, Meniere Disease (MD), no-Meniere and Benign Paroxismal
Positioning Vertigo (BPPV).
Material and Methods
We studied 52 patients affected with cochleo-vestibular disturbances subdivided
into two groups of out-patients:
• Definite unilateral Meniere (Men): 12 subjects ( 8 males and 4.females, mean age
41,6.yy) according to international AOO-HNS 1995 diagnostic criteria [7]
• No-Meniere (No-Men): 14 subjects (6 males and 8 females, mean age 44,7.yy)
affected with unilateral cocleo-vestibular impairment
A third group of subjects have been considered, as a “normal” group, 13 patients
(8 females and 5 males, mean age 45,5 yy) affected with Benign Paroxismal Positioning Vertigo (BPPV) with cochlear involvement
All of them underwent to a complete audio-vestibular investigation by the same
audiologist (MV)) according to international acceptd protocol [8] . Diagnosis was
posed by the same otoneurologist (A.DC).
ECD was performed by the same specialist (BPM) and two test were performed:
1) Arteries test. Flow of vertebral artery (VA) of both sides have been investigated both
Chronic Cerebrospinal Venous Insufficiency (CCSVI) IN Meniere Disease
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in static and dynamic positions in order to evaluate the variations of arterial flow due
to head and neck movements. The flow, static and dynamic, of the cochlear artery
(CA) of both sides have been investigated, too, according to the method described by
Belcaro and Nicoalides [9] with the patient is completely lying flat on his back in the
most comfortable way, with a low pillow, searching absolute neck relaxation to avoid
possible muscular contraction. The Duplex access to “find” the cochlear arterial system
may differ from one patient to another and should be carried out from a posterior
and anterior projection of the ear and “working” a lot on the machine’s capacities. It
isn’t only a matter of what is seen but also of what is heard and transformed into a
spectral flow analysis The blood flow exam is mostly based on the CW scale more
than on the imaging itself because of the small vessel dimensions.
ECD was considered abnormal when the vertebral and/or the cochelar artery
presented a reduced flow in static and/or dynamic conditions at the same side of the
affected ear at least of 50% with respect of the controlateral side
2) Veins test, according to the protocol described by Zamboni et al. [1]. The CCSVI
protocol exam at 00° and 90° was carried out on informed and willing patients
according to CCSVI diagnosis procedure.
ECD was considered abnormal when at least two out five of Venous Haemodynamic Insufficiency Severity Score (VHISS) criteria were satisfied.
Results were discussed with an independent well-trained otoneurologist (HA).
MEN vs no-MEN vs BPPV findings were compared by means of Student t-test and
considered as significant at 0.005 p level.
Results
Asymmetrical artherious flow in VA or CA was revealed in 2 Men 9 no-Men and
1 BPPV, respectively 12,5 - 60,7 - and 8,6 %. Differences between Men and No-Men
and between each of this group with respect to BPPV were highly significant (p<0.001).
Asymmetrical venous flow in IJV or VV was detected in 9 patients in MEN group
and in 4 in no-MEN and 2 BPPV, respectively 79 - 28,5 and 13 %. Differences
between Men and No-Men and between each of this group with respect to BBV were
highly significant (p<0.001)
Tab.1: Figure shows the distribution of ECD abnormal tests in the three groups of patients. Artherious abnormalities are significant more represented in no-Men, venous abnormalities in Men, normal results in BPPV
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Alpini D.C., Bavera P.M., Hahn A., Mattei V.
Conclusions
Meniere’s disease is a chronic illness that affects a substantial number of patients
every year worldwide. The disease is characterised by intermittent episodes of vertigo
lasting from minutes to hours, with fluctuating sensorineural hearing loss, tinnitus,
and aural pressure. The primary histopathological correlate is endolymphatic hydrops.
Paparella [10] used the notion of “lake-river-pond” to explain the occurrence of malabsorption of endolymph leading to hydrops. This notion describes the endolymphatic sac
as a pond, with the vestibular aqueduct (the river) connecting it to the endolymphatic
fluid space that is like a lake. When there is an obstruction near the endolymphatic
sac or duct, a backlog of endolymphatic fluid is created, leading to hydrops.
The vertebral artery is a major artery in the neck, It branches from the subclavian
artery, where it arises from the postero-superior portion of the subclavian artery. It ascends
thought the foramina of the transverse processes of the sixth cervical vertebrae. Then, it
winds behind the superior articular process of the atlas. It enters the cranium through the
foramen magnum where it unites with the opposite vertebral artery to form the basilar
artery (at the lower border of the pons). At the junction between the medulla oblongat a
and the pons two vertebral arteries joint into the basilar artery, forming the so-called
the vertebrobasilar system, which supplies blood to the posterior part of circle of Willis
and anastomoses with blood supplied to the anterior part of the circle of Willis from the
internal carotid arteries.
The basilary artery ascends in the central gutter (sulcus basilaris) inferior to the
pons and divides into the posterior cerebral arteries and the superior cerebellar artery. From the basilar artery arises the anterior inferior cerebellar artery (supplying
the superior and inferior aspects of the cerebellum), as well as smaller branches for
the supply of the pons (the pontine branches). In under 15% of people the basilar
artery gives rise to the labyrinthine artery while, generally, the labyrinthine artery
(then subdivide into cochlear and vestibular arteries), is a long slender branch of the
anterior inferior cerebellar artery (more or less 85% cases) or basilar artery (<15%
cases), arises from near the middle of the artery; it accompanies the vestibulocochlear
nerve through the internal acoustic meatus, and is distributed to the internal ear.[11]
The veins of the vestibule and semicircular canals accompany the arteries, and,
receiving those of the cochlea at the base of the modiolus, unite to form the internal
auditory veins (or veins of labyrinth) which end in the posterior part of the superior
petrosal sinus or in the transverse sinus. [12] The common modiolar vein enters the
bony channel immediately adjacent to the aqueduct to become the vein of the cochlear aqueduct which in turn drains via the inferior petrous sinus into the Internal
Jugular Veins (IJVs). Injury or occlusion of this vessel would be particularly significant since it is widely believed to provide virtually the entire venous drainage of
the cochlea. [13] The cochlear aqueduct and the internal auditory canal communicate
with the subarachnoidal space; in the guinea pig model, an occlusion of the veins of
the cochlear aqueduct results in an increase of perilympatic endolymphatic pressure,
a decrease of cochlear blood flow and endolymphatic potential. Furthermore, since
many of the venous vessels in the scala tympani have little or no bony covering and
are essentially exposed to the perilymphatic space, the venous system is a route of
entry for the cells participating in the inner ear inflammatory process. [14]
Another interesting point is that the blood leaves the brain by using the back
propulsion of the residual arterial pressure (vis a tergo), complemented by antero-
Chronic Cerebrospinal Venous Insufficiency (CCSVI) IN Meniere Disease
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grade respiratory mechanisms (vis a fronte). The latter consist of the thoracic pump
increased venous outflow during inspiration: the increase of negative thoracic pressure
improves the aspiration of blood toward the right atrium. In addition to vis a tergo
and vis a fronte, postural mechanisms play a fundamental role in ensuring a correct
cerebral venous return.
The pattern of cerebral venous drainage changes, even under physiological conditions, is thus depending on the body position. In the prone or supine position, the
outflow through the IJVs is favoured, whereas passing to the upright position transfers
most of the encephalic drainage to the vertebral veins. [15]
In recent years, together with gradual improvement of Duplex machines, interest
has been gained in searching anatomical diagnostic areas that appear both critical and
challenging, for the operator and the apparatus that is being employed. The vascular
anatomy of the cochlea is the most unusual and probably the most difficult to evaluate.
The blood flow is segmentally, centrifugally, and centripetally arranged, with many
spirally perpendicular vessels shunted in, suggesting good possibilities for variations
of circulation. There is a pronounced apical simplification of the vascular pattern
probably more than corresponding to the decreasing volume of the turns. The blood
supply of the scala vestibuli is mainly arterial; that of the scala media is capillary
or by-pass, and that of the scala tympani is venous. For this last reason, and more
recently, the Duplex examination is carried out also in an upright sitting position,
always kept comfortable for the neck, so to register the difference of the venous
outflow according to postural pressure and different breathing activity.
The cochlear artery, often correlated with severe sudden deafness [16,17], appears
one of the most difficult vascular exams to carry out. Moreover, this exam isn’t easy
to transform into a standard path, like for example the most common Duplex exam of
the carotid arteries, because the anatomical access is extremely difficult and varies from
one patient to another, or even gender since women generally have a smaller structure
and window access to the area.The two sides appear very much like a stereophonic
Hi-Fi apparatus and so both should register similar bloodstream haemodynamic flow.
An important predominance of one side upon the other clearly is abnormal.
The most frequent abnormalities detected with the internal jugular veins along
their extra-cranial way. In case of a pathologically slow jugular outflow and secondary
venous hypertension [18], other veins have to increase their normal activity of venous
drainage and Duplex is the only possible exam, up till now, that allows imaging in
lying and sitting positions and also requires patient interaction. The vertebral veins
appear to be among those that increase, as far as it is anatomically possible, the venous
drainage. Not rarely, also the vertebral outflow appears insufficient and, consequently,
venous hypertension can be present also in this segment.
It is now being demonstrated that venous outflow problems are present in several progressive neurologic diseases. Symptoms may vary in percentages but, those
regarding postural and/or listening problems seem to be always more frequent in all
the diseases. [4]
The echo-colour Doppler (ECD) is the ideal tool for dynamic assessment of both
cerebral arterial inflow and venous outflow. In our patients ECD indicated dynamic
abnormalities of blood venous cerebro-spinal drainage quite exclusively in Meniere
Disease and arterial alterations in no-Men group and normal cerebro-cephalic pattern
in BPPV.
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Alpini D.C., Bavera P.M., Hahn A., Mattei V.
On the basis of our results we can suggest that blood outflow could play a pathogenetic role in Meniere Disease [19] while in no-Meniere Disease cochlea-vestibular
damage may be mainly sustained by a reduced blood inflow. Substantial normalities
of ECD in BPPV confirm that BPPV is a vestibular dysfunction and not a lesion of
he inner ear, at least in the majority of the cases.
To the best of the authors’ knowledge, the association between Meniere Disease
and CCSVI has never been published.
CCSVI might explain the anatomical background which provides a predisposing
factor for cochlea-vestibular diseases, in general, and Meniere Disease, particularly.
ECD if aimed to detect specific blood flow abnormalities is a specific tool for the
pathogenetic differential diagnosis in cochlea-vestibular disorders
Further studies in larger groups of patients are needed to investigate the exact
mechanisms and correlation between inner ear diseases and Cerebrospinal ArteroVenous flow abnormalities.
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